Infantile myofibromatosis treated by mandibulectomy and staged reconstruction with submental flap and free fibula flap: a case report
Abstract Background Infantile myofibromatosis is the most common benign fibrous tumor in infants. Three different types have been reported in the literature. The most commonly affected areas are the head, the neck and the trunk. Our patient showed a very high level of mandibular destruction resistan...
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| Format: | Article |
| Language: | English |
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SAGE Publishing
2019-03-01
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| Series: | Journal of Otolaryngology - Head and Neck Surgery |
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| Online Access: | http://link.springer.com/article/10.1186/s40463-019-0333-z |
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| author | Alexandra Maby Benoit Guay François Thuot |
| author_facet | Alexandra Maby Benoit Guay François Thuot |
| author_sort | Alexandra Maby |
| collection | DOAJ |
| description | Abstract Background Infantile myofibromatosis is the most common benign fibrous tumor in infants. Three different types have been reported in the literature. The most commonly affected areas are the head, the neck and the trunk. Our patient showed a very high level of mandibular destruction resistant to all mandibular sparing treatment strategies requiring segmental mandibulectomy and complex reconstruction. Case presentation We describe a rare case of multicentric infantile myofibromatosis with mandibular bone destruction. The treatment required a succession of chemotherapy, a subtotal transoral resection and a hemi-mandibulectomy. The mandibular reconstruction was staged with initial bridging titanium plate with a submental flap, followed later by a fibula free flap. Conclusion Mandibular involvement by myofibromatosis is rare, and the extend of bone destruction and reconstruction make this case unique. To our knowledge, this is the only reported case of fibula free flap mandibular reconstruction in a patient with infantile myofibromatosis , as well as one of the youngest reported submental island flaps for any pathology. We describe the clinical presentation and management, including relevant imaging, histopathology, medical and surgical treatment as well as a review of relevant literature. |
| format | Article |
| id | doaj-art-cca04955f90e47d6b7c8a18b7ed709c0 |
| institution | Kabale University |
| issn | 1916-0216 |
| language | English |
| publishDate | 2019-03-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Journal of Otolaryngology - Head and Neck Surgery |
| spelling | doaj-art-cca04955f90e47d6b7c8a18b7ed709c02025-02-03T00:22:58ZengSAGE PublishingJournal of Otolaryngology - Head and Neck Surgery1916-02162019-03-0148111010.1186/s40463-019-0333-zInfantile myofibromatosis treated by mandibulectomy and staged reconstruction with submental flap and free fibula flap: a case reportAlexandra Maby0Benoit Guay1François Thuot2Département d’ophtalmologie et d’oto-rhino-laryngologie – chirurgie cervico-faciale, Faculté de Médecine, Université LavalDépartement d’ophtalmologie et d’oto-rhino-laryngologie – chirurgie cervico-faciale, Faculté de Médecine, Université LavalDépartement d’ophtalmologie et d’oto-rhino-laryngologie – chirurgie cervico-faciale, Faculté de Médecine, Université LavalAbstract Background Infantile myofibromatosis is the most common benign fibrous tumor in infants. Three different types have been reported in the literature. The most commonly affected areas are the head, the neck and the trunk. Our patient showed a very high level of mandibular destruction resistant to all mandibular sparing treatment strategies requiring segmental mandibulectomy and complex reconstruction. Case presentation We describe a rare case of multicentric infantile myofibromatosis with mandibular bone destruction. The treatment required a succession of chemotherapy, a subtotal transoral resection and a hemi-mandibulectomy. The mandibular reconstruction was staged with initial bridging titanium plate with a submental flap, followed later by a fibula free flap. Conclusion Mandibular involvement by myofibromatosis is rare, and the extend of bone destruction and reconstruction make this case unique. To our knowledge, this is the only reported case of fibula free flap mandibular reconstruction in a patient with infantile myofibromatosis , as well as one of the youngest reported submental island flaps for any pathology. We describe the clinical presentation and management, including relevant imaging, histopathology, medical and surgical treatment as well as a review of relevant literature.http://link.springer.com/article/10.1186/s40463-019-0333-zInfantile myofibromatosisMyofibromaMandibular reconstructionSubmental island flapFibula free flap |
| spellingShingle | Alexandra Maby Benoit Guay François Thuot Infantile myofibromatosis treated by mandibulectomy and staged reconstruction with submental flap and free fibula flap: a case report Journal of Otolaryngology - Head and Neck Surgery Infantile myofibromatosis Myofibroma Mandibular reconstruction Submental island flap Fibula free flap |
| title | Infantile myofibromatosis treated by mandibulectomy and staged reconstruction with submental flap and free fibula flap: a case report |
| title_full | Infantile myofibromatosis treated by mandibulectomy and staged reconstruction with submental flap and free fibula flap: a case report |
| title_fullStr | Infantile myofibromatosis treated by mandibulectomy and staged reconstruction with submental flap and free fibula flap: a case report |
| title_full_unstemmed | Infantile myofibromatosis treated by mandibulectomy and staged reconstruction with submental flap and free fibula flap: a case report |
| title_short | Infantile myofibromatosis treated by mandibulectomy and staged reconstruction with submental flap and free fibula flap: a case report |
| title_sort | infantile myofibromatosis treated by mandibulectomy and staged reconstruction with submental flap and free fibula flap a case report |
| topic | Infantile myofibromatosis Myofibroma Mandibular reconstruction Submental island flap Fibula free flap |
| url | http://link.springer.com/article/10.1186/s40463-019-0333-z |
| work_keys_str_mv | AT alexandramaby infantilemyofibromatosistreatedbymandibulectomyandstagedreconstructionwithsubmentalflapandfreefibulaflapacasereport AT benoitguay infantilemyofibromatosistreatedbymandibulectomyandstagedreconstructionwithsubmentalflapandfreefibulaflapacasereport AT francoisthuot infantilemyofibromatosistreatedbymandibulectomyandstagedreconstructionwithsubmentalflapandfreefibulaflapacasereport |